Figure 6.1
Crusted plaque on the right cheek of the patient
Based on the case description and photograph, what is your diagnosis?
1.
Localized pemphigus vulgaris
2.
Localized cicatricial pemphigoid
3.
Impetigo
4.
Dermatophytosis
Microscopic examination of the skin biopsy specimen revealed intraepidermal acantholysis supporting the diagnosis of pemphigus vulgaris (PV). Direct immunofluorescence showed intercellular staining with IgG and C3 in the epidermis.
Diagnosis
Localized Pemphigus Vulgaris
The first presentation of PV in this patient was multiple blisters and erosions on the face, scalp and upper trunk and erosions on the lip and oral mucosa. At that time, the patient was treated with systemic prednisolone and azathioprine. She had good response and prednisolone was tapered. Generalized PV recurred several times on low dose prednisolone. Recurrences were treated with increase in the dose of prednisolone. After a few years, a 6 × 4 cm crusted plaque surrounded by erythema appeared on her right cheek with the involvement of lower eyelid. Intralesional triamcinolone was injected several times with the diagnosis of localized PV. The lesion had partial improvement but recurred at the same site a couple of times.
Discussion
PV is an autoimmune blistering disease with autoantibodies against the cell surface of keratinocyte. Initial involvement of oral mucosa is a well-known feature of the disease seen in 50–70 % of patients. These lesions are usually followed by the onset of more disseminated ones, on the mucosa or on the skin, after a time lapse ranging from weeks to months [1].
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